Clinical characteristics of hemophagocytic lymphohistiocytosis following Kawasaki disease: differentiation from recurrent Kawasaki disease
Blood Research
; : 254-257, 2013.
Article
in English
| WPRIM (Western Pacific)
| ID: wpr-25186
Responsible library:
WPRO
ABSTRACT
BACKGROUND:
Our aim was to investigate the clinical pattern of hemophagocytic lymphohistiocytosis following Kawasaki disease (HLH-KD), to enable differentiation of HLH from recurrent or refractory KD and facilitate early diagnosis.METHODS:
We performed a nationwide retrospective survey and reviewed the clinical characteristics of patients with HLH-KD, including the interval between KD and HLH, clinical and laboratory findings, treatment responses, and outcomes, and compared them with historical data for both diseases.RESULTS:
Twelve patients with HLH-KD, including 5 previously reported cases, were recruited. The median age was 6.5 years (range, 9 months-14.7 years). Eight patients were male and 4 were female. The median interval between the first episode of KD and the second visit with recurrent fever was 12 days (3-22 days). Of the 12 children, 2 were initially treated with intravenous IgG (IVIG) for recurrent KD when they presented at the hospital with recurrent fever. Eventually, 10 children received chemotherapy under an HLH protocol and 2 received supportive treatment. Two patients died of combined infections during chemotherapy, 1 was lost to follow up, and 9 remain alive. The overall survival rate at 4 years was 81.1% with a median follow up of 45.1 months.CONCLUSION:
A diagnosis of HLH-KD should be considered when symptoms similar to recurrent KD develop within 1 month of the first episode of KD. Our findings will help physicians differentiate between HLH and the recurrent form of KD.
Full text:
Available
Health context:
SDG3 - Health and Well-Being
/
SDG3 - Target 3.4 Reduce premature mortality due to noncommunicable diseases
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SDG3 - Target 3.2 Reduce avoidable death in newborns and children under 5
Health problem:
Target 3.4: Reduce premature mortality due to noncommunicable diseases
/
Target 3.2: Reduce avoidable death in newborns and children under 5
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Cardiovascular Disease
/
Skin Diseases
/
Noncommunicable Diseases
Database:
WPRIM (Western Pacific)
Main subject:
Immunoglobulin G
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Survival Rate
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Retrospective Studies
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Follow-Up Studies
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Early Diagnosis
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Diagnosis
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Drug Therapy
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Lymphohistiocytosis, Hemophagocytic
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Lost to Follow-Up
/
Fever
Type of study:
Diagnostic study
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Practice guideline
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Observational study
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Prognostic study
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Risk factors
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Screening study
Limits:
Child
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Female
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Humans
/
Male
Language:
English
Journal:
Blood Research
Year:
2013
Document type:
Article